Case of angioimmunoblastic T-cell lymphoma presenting as peripheral and bone marrow plasmacytosis: A diagnostic conundrum.

ABSTRACT: Angioimmunoblastic T-cell lymphoma (AITL), a subtype of peripheral T-cell lymphoma (PTCL), is associated with unique clinical, morphological, and immunohistochemical features. The peripheral circulation might show presence of an occasional reactive plasma cell but significant plasmacytosis masquerading as plasma cell leukemia is rare. We report a case of AITL in a 42-year-old male, who presented with two-month history of generalized lymphadenopathy. On investigations, he had hypergammaglobulinemia and plasmacytosis in the peripheral blood and bone marrow masquerading as plasma cell leukemia. Immunohistochemistry and serum protein electrophoresis revealed polyclonal nature of plasma cells. Diagnosis of AITL was made on cervical lymph node biopsy. This case highlights the diagnostic challenge faced due to heterogeneity in the clinical presentation and pathological findings and to alert the clinician so that timely accurate diagnosis can be made to initiate the treatment.

Optochiasmatic Cavernomas: Updated systematic review and proposal of a novel classification with surgical approaches.

Cavernomas are histologically benign vascular malformations found at different sites in the brain. A rare site for such cavernomas, however, is the anterior optic pathway, comprising the optic nerve, chiasma, and optic tract-called optochiasmatic cavernomas (OCC). These lesions usually present with sudden onset or progressive vision loss, headache, and features mimicking pituitary apoplexy. In this paper, we describe a case of OCC operated at our center. We carry out an updated review of literature depicting cases of OCC, their clinical presentation, management, and postoperative complications. We also propose a novel classification system based on lesion location and further analyze these cavernoma types with respect to the surgical approach used and visual outcome. A 30-year-old lady had presented with a 3-week history of progressive bilateral vision loss and headache. Based on imaging, she was suspected to have a cavernous angioma of the chiasma and left optic tract. Due to progressive vision deterioration, the lesion was surgically excised using pterional craniotomy. Postoperatively, her visual symptoms improved, but she developed diabetes insipidus. Clinical and radiological follow-up has been done for 18 months after surgery. A total of 81 cases have been described in the literature, including the present case. Chiasmal apoplexy is the most common presentation. Surgical excision is the standard of care. Our analysis based on lesion location shows the most appropriate surgical approach to be used for each cavernoma type. Visual outcome correlates with the preoperative visual status. Visual outcome is good in patients presenting with acute chiasmal apoplexy, and when complete surgical excision is performed. The endonasal endoscopic approach was found to provide the best visual outcome. In addition to preoperative visual status, complete surgical excision predicts favorable visual outcomes in OCC. Our proposed classification system guides the appropriate surgical approach required for a particular location of the cavernoma.

A Rare Sparkle: A Case of Calcified Kidneys in a Young Infant With Renal Failure.

Primary hyperoxaluria-1 (PH1) is an autosomal recessively inherited rare genetic condition due to the deficiency of the hepatic enzyme alanine:glyoxylate aminotransferase which leads to high systemic levels of oxalate and subsequently, early end-stage renal disease and death. Here, we present a case of a three-month-old male infant who presented with loose stools, reduced oral intake, and decreased activity for 12-13 days along with edema and a peeling rash on cheeks, lips, and genitalia. During the entire duration of the inpatient stay, the child was oligoanuric. Kidney ultrasound (USG) was suggestive of bilateral hyperechoic kidneys with increased cortical echogenicity and a computed tomography scan showed bilateral diffusely calcified renal cortices with well-preserved renal architecture. A diagnosis of "oxalate nephropathy" was made from renal biopsy and genetic testing confirmed it to be "primary hyperoxaluria-1". The child was initially managed conservatively, and then peritoneal dialysis was done, following which the child was shifted to intermittent hemodialysis.

Correlation between androgen and estrogen receptor expression and clinicopathologic features in carcinoma urinary bladder.

INTRODUCTION: The molecular mapping of cancers by the Cancer Genome Atlas Project has accelerated the quest for new therapeutic targets for urinary bladder cancer, including sex steroid receptors. Previous studies have demonstrated conflicting results on their relationship with bladder cancer, and there is sparse data on their expression in the Indian population. The aim of our study is to examine the expression of androgen receptors (AR) and estrogen receptors (ERα and ERß) in patients with bladder cancer and their correlation with clinicopathologic features. MATERIALS AND METHODS: In this retrospective cohort study, a total of 132 patients, who were surgically managed for urinary bladder mass by transurethral resection or radical cystectomy in our institute, with transitional cell carcinoma on histopathology and with at least two years of follow-up were included. Their demographic and treatment details were obtained, histopathology blocks were retrieved and immunohistochemical staining for androgen and estrogen receptors was performed. Then, the relationship between their expression and clinicopathologic features was studied. RESULTS: A total of 3.79% of patients showed estrogen receptor alpha positivity, 51.52% estrogen receptor beta positivity and 63.64% androgen receptor positivity. No statistically significant correlation was found between age of patients (p = 0.75/0.52/0.87), tumour stage and grade (0.71/0.3/0.21), pathological variant (p = 1/0.58/0.38) and overall survival (p = 0.70/0.052/0.45 for NMIBC and p = 0.82/0.36/0.22) and estrogen receptor alpha, estrogen receptor beta and androgen receptor-positive status, respectively. Estrogen receptor beta positivity was significantly higher in patients with unifocal (p = 0.015) and small tumours (< 5 cm) (p = 0.03), and its expression was associated with better disease-free survival (DFS) (p = 0.046) in patients of non-muscle invasive bladder cancer (NMIBC). CONCLUSION: Our study has the largest sample size conducted on Indian population with results differing from previous studies conducted on western population. Estrogen receptor beta expression was significantly associated with small unifocal tumours and better DFS. Estrogen receptor alpha and androgen receptor expression were not found to be associated with the clinicopathologic features of the study population.

Gangliocytic Paraganglioma of the Duodenum: A Masquerader.

Background: Gangliocytic paraganglioma (GP) is a rare tumor that most commonly arises from the duodenum and is characterized pathologically by 3 cell types: epithelioid, spindle, and ganglion cells. GP is often difficult to differentiate from a neuroendocrine tumor on the basis of preoperative imaging, and the diagnosis is based on final histopathologic and immunohistochemical analysis. Case Report: We report the case of a 28-year-old male who presented with pain in the abdomen, bilious vomiting, and weight loss. Imaging showed a mass involving the first and second part of the duodenum that was likely a neuroendocrine or gastrointestinal stromal tumor. He underwent robotic-assisted pancreatoduodenectomy, and the final pathology report identified GP with lymph node metastasis. The patient was doing well at 1-year follow-up. Conclusion: GP is often a histologic surprise as most cases are diagnosed in postoperative histopathology. While GP has a more benign course than a neuroendocrine tumor, radical surgical resection is warranted in cases of diagnostic dilemma, suspicion of malignancy, or lymph node metastasis. Robotic-assisted pancreatoduodenectomy is a feasible option.

Renal Allograft Malakoplakia Presenting as a Pseudotumoral Lesion.

Malakoplakia is an uncommon inflammatory disease that can involve many organ systems but is often encountered in the urogenital tract. Kidney allograft malakoplakia is even rarer and can have a diffuse parenchymal or a pseudotumoral presentation. We describe a case of grafi malakoplakia in an adult female, who presented with dull aching pain in the right loin, fever, and vomiting. Ultrasonography of the kidney graft showed a heterogeneous lesion (2.6 cm × 2.9 cm), raising suspicion of primary or metastatic renal tumors. The diagnosis was established after a histopathological examination of the kidney biopsy. This pseudotumoral presentation of malakoplakia can mimic renal cell carcinoma, lymphoma, fungal infections, or tuberculosis. It is essential to perform a biopsy for establishing the diagnosis.

Ultrastructural Changes in Autopsy Tissues of COVID-19 Patients.

INTRODUCTION: The COVID-19 pandemic resulted in substantial morbidity and mortality across the world. The prognosis was found to be poor in patients with co-morbidities such as diabetes, hypertension, interstitial lung disease, etc. Although biochemical studies were done in patient samples, no study has been reported from the Indian subcontinent about ultrastructural changes in the vital organs of COVID-19 patients. The present study was, therefore, conducted to understand the ultrastructural changes in the lung, liver, and brain of the deceased patients. METHODS: The present study was conducted on samples obtained from reverse transcription-polymerase chain reaction (RT-PCR)-positive patients who were admitted to a tertiary care hospital in Western India. Core needle biopsies were done in eight fatal cases of COVID-19. The samples were taken from the lungs, liver, and brain and subjected to light microscopy, immunohistochemistry (IHC), and transmission electron microscopy (TEM). Clinical details and biochemical findings were also collected.  Results: The study participants included seven males and one female. The presenting complaints included fever, breathlessness, and cough. Light microscopy revealed diffuse alveolar damage in the lungs. Further, a positive expression of SARS-CoV-2 nucleocapsid protein was observed in the pulmonary parenchyma of five patients. Also, the TEM microphotograph showed viral particles of size up to 80nm localized in alveolar epithelial cells. However, no viral particles were found in liver or brain samples. In the liver, macrovesicular steatosis and centrizonal congestion with loss of hepatocytes were observed in light microscopy. CONCLUSION:  This is the first study in the Indian population showing the in-situ presence of viral particles in core biopsies from fatal cases of COVID-19. As evident from the results, histology and ultrastructural changes in the lung correlated with the presence of viral particles. The study revealed a positive correlation between the damage in the lungs and the presence of viral particles.

Mortality and Its Predictors in COVID-19 Patients With Pre-existing Interstitial Lung Disease.

Background The data on the impact of coronavirus disease 2019 (COVID-19) on interstitial lung disease (ILD) is still limited. To the best of our knowledge, there has been no study from India to date to assess the impact of COVID-19 in patients with preexisting ILD. We undertook this study to assess the clinical outcome of ILD patients admitted to our hospital with COVID-19. Methods In this retrospective observational study, records of reverse transcription polymerase chain reaction (RT-PCR)-confirmed COVID-19 patients with preexisting ILD who were admitted to the hospital in the period from May 1, 2020, to April 30, 2021, were obtained from the hospital database. The clinical outcomes of the patients were recorded. Univariate analysis was performed to find relation between various predetermined risk factors for mortality and those with significant p values (p<0.05) were subjected to multiple logistic regression to determine independent risk factors. Results In our study of 28 patients, the overall mortality was 35.7%. On comparing the parameters associated with increased mortality, there was no effect of age, gender, comorbidities, type of ILD, CT thorax findings on diagnosis, use of corticosteroids and antifibrotics in the past, spirometric findings on mortality. On multivariate analysis, the significant parameters were interleukin 6 (IL-6), p=0.02, OR=1.020 (1.006-1.043) and D-dimer, p=0.04, OR=2.14 (5.55-1.14). Conclusion COVID-19 in patients with pre-existing ILD has a comparatively higher mortality. D-dimer and IL-6 are significant predictors of mortality in ILD patients infected with COVID-19.

Risk Stratification of Pleural Fluid Cytology Based on the International System for Reporting Serous Fluid Cytology in a Tertiary Care Centre.

BACKGROUND: The International Academy of Cytology and the American Society of Cytopathology proposed the international system for reporting serous fluid cytology (TISRSFC) in 2019 to form uniform reporting terminologies for fluid cytology. This system defines a spectrum of diagnostic categories to be used in daily clinical practice. Fluid cytology is a widely accepted, cost-effective, minimally invasive earliest diagnostic method for the investigation that aids management decisions. The present study aims to reclassify pleural fluid cytology and calculate the risk of malignancy (ROM) for each diagnostic category. MATERIAL AND METHODS: It is a retrospective 2 years observational study comprising 690 pleural fluid specimens. Retrospective reviews were performed, and cases were reclassified into five categories as per TISRSFC. Immunohistochemistry was applied whenever needed. Cytological diagnosis was correlated with respective histopathology and/or clinical and/or radiological diagnosis. ROM was calculated for all diagnostic categories. RESULTS: In the present study of 690 cases, 7.97% were non-diagnostic (ND), 84.1% cases were negative for malignancy (NFM), 0.87% were atypia of undetermined significance (AUS), 1.16% were suspicious for malignancy (SFM), and 5.94% were malignant (MAL). Cell blocks were prepared in 33 (4.8%) cases and immunohistochemistry was applied in 7 cases. The commonest site for pleural fluid metastasis was the lungs, accounting for 4.64% (32/690) cases. Further, ROM was calculated for all diagnostic categories as follows: (1) ND: 30.9%, (2) NFM: 12.9%, (3) AUS: 100%, (4) SFM: 100%, and (5) MAL: 90.2%. CONCLUSION: Cytological examination of pleural fluids is an accurate, prompt, and affordable technique. This standardized ISRSFC reporting system will maintain uniformity and reproducibility in reporting, leading to improved clinical decision-making of pleural fluids.

Umbilicated papulonodules on the elbows with purpuric papules on the fingers.

This case describes an unusual cutaneous presentation. Our patient had purpuric papules on the fingers and umbilicated nodules over the elbows, and interestingly, both lesion types showed similar histopathological features of necrobiotic granuloma with neutrophils.

Cervical Thymic Cyst with parathyroid tissue - a diagnostic conundrum.

Cervical thymic cysts are relatively rare benign cystic lesions that tend to be diagnosed clinically as branchial cysts, which usually present as painless, enlarging neck masses. They can occur anywhere along the normal path of descent of thymic primordia from the angle of the mandible to the sternal notch, with mediastinal extension observed in approximately 50% of cases. They are usually seen in the first decade of life on the left side with a male predominance. Here we report a case of a 15-year-old boy who presented to the hospital with left-sided neck swelling for about 2 months. The neck's contrast-enhanced computed tomography (CECT) revealed a large, well-defined cystic swelling in the left neck region, showing peripheral enhancement, seen from the submandibular region to the superior mediastinum extending into the retrosternal region. Direct fine needle aspiration (FNA) was done, which showed a benign lesion with inflammatory and cystic characteristics, leading to the possibility of a branchial cyst. The cyst was completely excised surgically. Histopathology showed a thymic cyst with parathyroid tissue. The presence of thymic tissue with Hassall's corpuscles is essential for the diagnosis. Knowledge of the clinical presentation, cyto-histological findings, and differential diagnosis of cystic cervical lesions in the pediatric population is important to diagnose this rare entity. Hence, though uncommon, when one comes across a cystic cervical region mass in children, a diagnosis of cervical thymic cyst should be kept in mind. Nonetheless, a definitive diagnosis depends on imaging findings as well as intraoperative findings and histopathological examination.

ALK-positive anaplastic large cell lymphoma of skeletal muscle masquerading as soft tissue sarcoma.

Anaplastic large cell lymphoma (ALCL) is a subtype of T cell non-Hodgkin's lymphoma and can present as an extranodal disease. Primary ALCL of skeletal muscle is rare. We report a case of ALK-positive ALCL involving the left thigh and anterior chest wall in a 12-year-old male child. The fine needle aspiration cytology from the lesion showed cellular smears with singly scattered and occasional clusters of large pleomorphic atypical cells. A diagnosis of poorly differentiated malignant neoplasm was rendered. On core biopsy, the histomorphological features posed a diagnostic challenge with a myriad of morphological mimickers. The diagnosis was established by excluding specific entities by relevant immunostains and confirming the diagnosis by strong expression of CD30 and ALK on immunohistochemistry. Fluorescence in-situ hybridisation confirmed the characteristic t(2:5) translocation. Presentation of ALCL with skeletal muscle involvement is uncommon, and the diagnosis relies on broadening the diagnostic possibilities and judicious use of immunohistochemical markers.